New York Times (May 2, 2011): A Thief That Robs the Brain of Language

Steve Riedner of Schaumberg, Ill., was a 55-year-old tool-and-die maker, a job that involves difficult mental calculations, and a frequent speaker at community meetings when he found himself increasingly at a loss for words and unable to remember numbers. He even began to have difficulty reading his own written comments.

The neurologist he consulted thought Mr. Riedner had suffered a stroke and for three years treated him with cholesterol-lowering medication. But instead of his language ability stabilizing or improving, as should happen following a stroke, it got worse.

A second neurologist concluded after further testing that Mr. Riedner might have a condition called primary progressive aphasia, or P.P.A., a form of dementia affecting the brain’s language center.

Having seen only one other case in his career, the neurologist referred Mr. Riedner and his wife, Mary Beth, to the Cognitive Neurology and Alzheimer's Disease Center at Northwestern University, whose director, Dr. M. Marsel Mesulam, is perhaps the world’s leading expert on this relatively rare disorder.

P.P.A. is a clinical syndrome, one of several forms of brain disease lost in the medical shadow of their much better known relative Alzheimer’s disease. While hardly as common as Alzheimer’s, P.P.A. is often misdiagnosed, and many patients like Mr. Riedner lose valuable time trying inappropriate and ineffective treatments. Though there is no cure, patients and families can learn ways to minimize the disabilities it causes.

Unlike Alzheimer’s, P.P.A. does not affect memory, at least not initially. It also tends to occur at younger ages, often in the late 50s, and affects twice as many men as women. While symptoms of Alzheimer’s are readily recognized by friends and relatives but not those affected, people with P.P.A. are painfully aware of their struggle to communicate, often long before it is apparent to others.